Case Study: Devic’s Disease and Sjogren’s Syndrome

I work in a hospital that serves an impoverished, medically underserved community. It is not uncommon to see patients who have not been treated for lingering health issues. 

In this case study, the patient is a 40-year-old Afro-Caribbean female with a history of cerebrovascular accident (CVA) with residual left upper extremity hemiparesis as well as Guillain-Barré syndrome requiring intubation and intravenous immunoglobulin (IVIG). On this admission, she presented with new onset of left foot drop with associated numbness and tingling; and, urinary incontinence.

Tests and Treatment

Physical exam revealed stable vital signs, decrease in sensation over the left side of her face and body with diminished muscle strength of the left lower extremity, left tongue deviation, and dry mucocutaneous membranes, especially eyes and oral cavity.

Her complete blood count and comprehensive panel were within normal limits. She was tested for serological markers, and found to have positive anti-nuclear antibody (ANA), SSA, and SSB. The imaging of the spine revealed hyperintense lesions in T12 and the conus medullaris (as shown below).

A lumbar puncture was performed revealing a pleocytosis in the cerebrospinal fluid (CSF) with atypical lymphocytes. The differential at this time included autoimmune diseases with neurological manifestations, malignancy, and infectious processes, such as Epstein-Barr Virus. Given her clinical presentation and workup including labs, imaging, and lumbar puncture, malignancy and infection was lowered on the differential once the flow cytometry showed no cells concerning for lymphoma/ leukemia.  Once that was decided, she completed a three-day course of Methylprednisolone (Solu-Medrol) 1,000 mg (pulse dose-steroids) and plasmapheresis with marked improvement in physical function.

Diagnosis

Anti-aquaporin-4 antibody (AQP4-Ab) was positive for neuromyelitis optica (Devic’s disease), a rare inflammatory autoimmune disease of the central nervous system, specifically of the spinal cord and optic nerves. The patient was continued on oral prednisone and started treatment with rituximab.

A bedside ultrasound of the parotid glands revealed non-homogenous glandular tissues supporting a diagnosis of Sjogren’s syndrome.

Both Neuromyelitis optica and Sjogren’s can cause severe CNS manifestations. It is conceivable that they are a spectrum of diseases on the same continuum. This also helps us to postulate that the presence of AQP4-Ab should be explored in all Sjogren’s patients, as it can alter their disease course. The treatment for both entities is similar and involves immunosuppression. However, it is imperative to tailor the treatment to each specific patient, as the disease has marked variations. 

Irina Litvin, D.O., is a physician and rheumatology fellow at SUNY Downstate in Brooklyn, N.Y.

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References:

Komai T, Shoda H, Yamaguchi K., et. al.  “Neuromyelitis optica spectrum disorder complicated with Sjogren syndrome successfully treated with tocilizumab: A case report, “ Modern Rheumatology. DOI: 10.3109/14397595.2013.861333. E-Pub 2013 Dec 9.

Waters P, Jarius S, Littleton E, Leite MI, et al. "Aquaporin-4 antibodies in neuromyelitis optica and longitudinally extensive transverse myelitis," Archives of Neurology. July 2008. DOI:  10.1001/archneur.65.7.913

Dr Romain Marignier, MD, Pascale Giraudon, PhD, et al., "Anti-aquaporin-4 antibodies in Devic’s neuromyelitis optica: therapeutic implications," Therapeutic Advances in Neurological Disorders. Sept. 2010. DOI:  10.1177/1756285610382478